Living with Pompe disease, Boston, MA
“My ultimate wish is to help people…I’ve learned since becoming immobile, when you need help, everything is either super-expensive or you don’t get it at all.”
Meet Princeton – husband, father and warrior
Princeton and his wife, Cassandra, look out at West Roxbury High School one last time as the setting sun falls to the west. The building is scheduled to be torn down.
They take a moment to reflect on the years they spent within those walls when Princeton was a competitive tennis player and an avid car enthusiast. This was all before Pompe disease – a rare genetic lysosomal storage disorder that progressively weakens muscles – caused this once fully able-bodied man to progressively lose control of his muscles over the last twenty years. Princeton’s Pompe is “late-onset,” and he considers himself extremely lucky despite having a rare genetic illness. “I’m in a better situation than a lot of people,” he relates. “Which is, in itself, disappointing to know that this is what Pompe can do. It’s scary because I don’t know if that’s ever going to happen to me, but I’m blessed to keep it where it’s at right now.”
Cassandra remembers meeting her future husband in the halls between classes. She recalls how Princeton used to walk. His gait was strange, although it never raised any red flags. “He stepped down like his feet were big and heavy. Almost like a penguin. It was cool. I was like, ‘You walk kind of funny, but whatever.’” This did little to deter Cassandra’s growing love. “I used to tell my friends, ‘I’m going to marry him someday.’”
The days of deterioration and discouragement
At eighteen, Princeton’s physical abilities first started to slip. When Princeton was twenty, he received the clinical diagnosis of Pompe disease, although it would take another two years for the genetic tests to confirm.
Princeton had spent years unsure of what medicine could do for him. “I heard ‘medicine’ and assumed that I was going to get better. It’s not until I actually got the medicine, went through the infusions and saw that I was still the same that I realized—this is something that’s not going to go away.” Enzyme replacement therapy for Pompe was the only treatment for the disease, but Princeton didn’t see the point. If it wasn’t going to cure him, why bother? Meanwhile, his physical condition deteriorated—walking became difficult—and he started using a cane.
Following a series of physical setbacks, Princeton transitioned to using a wheelchair full-time. His life slowed down and depression took hold. The realities of living with limited mobility were making it harder to commute to the hospital for needed infusions. He had to rely on an inconsistent paratransit van and if it didn’t show, it would cost a few hundred dollars to make the trip via an accessible taxi. More and more infusion-days passed by as Princeton waited for more vans that never came.
The Pompe warrior battles on
One day, after yet another missed infusion, Princeton’s spirit broke. He turned to the internet to express himself and ask for help. The response to his video was moving and inspired Princeton to create a GoFundMe campaign to raise money for a vehicle that could suit his needs. Though page only raised a couple hundred dollars, it created the momentum he needed. Through hard work and saving, he and Cassandra were finally able to buy a Dodge minivan complete with ramps, a swiveling driver’s seat, and hand-controls for driving.
Getting a set of wheels was life-changing. With reliable transportation, Princeton started school again, studying psychology at UMASS and on top of that, he re-entered the workforce working at a local call center.
Princeton continues to make YouTube videos and has developed a passion for video production, a skillset he now shares with this youngest son, Jovanni. It has helped him connect to the larger Pompe community through sharing stories, realities, frustrations, and grief. “It’s good to know that you’re not alone,” he says. “Someone’s going through the exact thing that you’re going through.”
On Princeton’s forearm is a tattoo that reads “Pompe Warrior.” As his physical strength continues to decline, his mental strength becomes the uplifting force. His battle is far from over but through self-determination and familial support, he continues on the upward tract.
“We’ve been through trauma together, and we work it out as a family together,” says Cassandra. “That’s what motivates us to keep going.”